No abstract

Primary immunodeficiencies (PIDs) are a group of congenital diseases of the immune system, which numbers more than 230 nosological entities associated with lost, decreased, or wrong function of its one or several components. Due to the common misconception that these are extremely rare diseases that occur only in children and lead to their death at an early age, PIDs are frequently ruled out by physicians of related specialties from the range of differential diagnosis. The most common forms of PIDs, such as humoral immunity defects, common variable immune deficiency, X-linked agammaglobulinemia, selective IgA deficiency, etc., are milder than other forms of PID, enabling patients to attain their adult age, and may even manifest in adulthood. Bronchopulmonary involvements are the most common manifestations of the disease in patients with a defect in humoral immunity. Thus, a therapist and a pulmonologist are mostly the first doctors who begin to treat these patients and play a key role in their fate, since only timely diagnosis and initiation of adequate therapy can preserve not only the patient's life, but also its quality, avoiding irreversible complications. Chest computed tomography changes play a large role in diagnosis. These are not specific for PID; however, there are a number of characteristic signs that permit this diagnosis to be presumed.

Common variable immunodeficiency (CVID) is the most prevalent clinically important form of primary immunodeficiencies (PID). CVID is characterized by a wide variability of complications which can affect almost all organs and systems. Infectious complications are more typical, but non-infectious complications, in particular granulomatous lymphocytic interstitial lung disease (GLILD), have more strong influence on the course, prognosis and severity of the disease and the patient’s quality of life. GLILD is a challenge not only from a diagnostic point of view (manifestations are often misdiagnosed and treated as sarcoidosis, tuberculosis, lymphoproliferative disease), but also in terms of treatment strategy selection. Rituximab - is one of the most promising methods of GLILD therapy, which showed its efficiency in limited clinical observations. A clinical case of successful treatment of GLILD associated with CVID, using rituximab on the background of a regular replacement immunotherapy with immunoglobulin for intravenous administration (IVIG) is described in this article.

Replacement immunotherapy with intravenous immunoglobulin (IVIG) has been used successfully for the treatment of primary immunodeficiency (PID) for over 60 years. During this time, a huge step forward in purification and manufacturing methods has been made. In addition IVIG therapy has been approved for other diseases and this number is steadily expanding causing an appearance of new IVIG on the pharmaceutical market. These drugs vary in composition, manufacturing technology and, as a consequence, in safety, administration rate, and efficacy. The new IVIG on the Russian market IG Vena was recently introduced. It has already proved its safety and efficacy abroad. This article presents the experience of using the drug IG Vena in adults with PID in immunopathology department of the Institute of Immunology FMBA Russia.